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NF1-Associated Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
1709893
•Concept ID:
C5236143
•
Neoplastic Process

Definition

A malignant peripheral nerve sheath tumor that is associated with NF1 gene inactivation and a history of neurofibromatosis type 1. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNF1-Associated Malignant Peripheral Nerve Sheath Tumor

Recent clinical studies

Etiology

Spatial Gene-Expression Profiling Unveils Immuno-oncogenic Programs of NF1-Associated Peripheral Nerve Sheath Tumor Progression.
Mitchell DK, Burgess B, White EE, Smith AE, Sierra Potchanant EA, Mang H, Hickey BE, Lu Q, Qian S, Bessler W, Li X, Jiang L, Brewster K, Temm C, Horvai A, Albright EA, Fishel ML, Pratilas CA, Angus SP, Clapp DW, Rhodes SD
Clin Cancer Res 2024 Mar 1;30(5):1038-1053. doi: 10.1158/1078-0432.CCR-23-2548. PMID: 38127282Free PMC Article
Circulating tumor DNA for malignant peripheral nerve sheath tumors in neurofibromatosis type 1.
Jones J, Cain S, Pesic-Smith J, Choong PFM, Morokoff AP, Drummond KJ, Dabscheck G
J Neurooncol 2021 Sep;154(3):265-274. Epub 2021 Sep 16 doi: 10.1007/s11060-021-03846-z. PMID: 34529228
Telomere alterations in neurofibromatosis type 1-associated solid tumors.
Rodriguez FJ, Graham MK, Brosnan-Cashman JA, Barber JR, Davis C, Vizcaino MA, Palsgrove DN, Giannini C, Pekmezci M, Dahiya S, Gokden M, Noë M, Wood LD, Pratilas CA, Morris CD, Belzberg A, Blakeley J, Heaphy CM
Acta Neuropathol Commun 2019 Aug 28;7(1):139. doi: 10.1186/s40478-019-0792-5. PMID: 31462295Free PMC Article
MEK inhibitors enhance therapeutic response towards ATRA in NF1 associated malignant peripheral nerve sheath tumors (MPNST) in-vitro.
Fischer-Huchzermeyer S, Dombrowski A, Wilke G, Stahn V, Streubel A, Mautner VF, Harder A
PLoS One 2017;12(11):e0187700. Epub 2017 Nov 13 doi: 10.1371/journal.pone.0187700. PMID: 29131833Free PMC Article
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406

Diagnosis

Spatial Gene-Expression Profiling Unveils Immuno-oncogenic Programs of NF1-Associated Peripheral Nerve Sheath Tumor Progression.
Mitchell DK, Burgess B, White EE, Smith AE, Sierra Potchanant EA, Mang H, Hickey BE, Lu Q, Qian S, Bessler W, Li X, Jiang L, Brewster K, Temm C, Horvai A, Albright EA, Fishel ML, Pratilas CA, Angus SP, Clapp DW, Rhodes SD
Clin Cancer Res 2024 Mar 1;30(5):1038-1053. doi: 10.1158/1078-0432.CCR-23-2548. PMID: 38127282Free PMC Article
Circulating tumor DNA for malignant peripheral nerve sheath tumors in neurofibromatosis type 1.
Jones J, Cain S, Pesic-Smith J, Choong PFM, Morokoff AP, Drummond KJ, Dabscheck G
J Neurooncol 2021 Sep;154(3):265-274. Epub 2021 Sep 16 doi: 10.1007/s11060-021-03846-z. PMID: 34529228
Systemic Options for Malignant Peripheral Nerve Sheath Tumors.
Hassan A, Pestana RC, Parkes A
Curr Treat Options Oncol 2021 Feb 27;22(4):33. doi: 10.1007/s11864-021-00830-7. PMID: 33641042
Non-Coding RNA and Tumor Development in Neurofibromatosis Type 1: ANRIL Rs2151280 Is Associated with Optic Glioma Development and a Mild Phenotype in Neurofibromatosis Type 1 Patients.
Tritto V, Ferrari L, Esposito S, Zuccotti P, Bianchessi D, Natacci F, Saletti V, Eoli M, Riva P
Genes (Basel) 2019 Nov 5;10(11) doi: 10.3390/genes10110892. PMID: 31694342Free PMC Article
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

Therapy

MEK Inhibition Synergizes with TYK2 Inhibitors in NF1-Associated Malignant Peripheral Nerve Sheath Tumors.
Borcherding DC, Amin NV, He K, Zhang X, Lyu Y, Dehner C, Bhatia H, Gothra A, Daud L, Ruminski P, Pratilas CA, Pollard K, Sundby T, Widemann BC, Hirbe AC
Clin Cancer Res 2023 Apr 14;29(8):1592-1604. doi: 10.1158/1078-0432.CCR-22-3722. PMID: 36799629Free PMC Article
Neurofibromatosis in the Era of Precision Medicine: Development of MEK Inhibitors and Recent Successes with Selumetinib.
Galvin R, Watson AL, Largaespada DA, Ratner N, Osum S, Moertel CL
Curr Oncol Rep 2021 Mar 15;23(4):45. doi: 10.1007/s11912-021-01032-y. PMID: 33721151Free PMC Article
Combined Targeting of AKT and mTOR Inhibits Proliferation of Human NF1-Associated Malignant Peripheral Nerve Sheath Tumour Cells In Vitro but not in a Xenograft Mouse Model In Vivo.
Schulte A, Ewald F, Spyra M, Smit DJ, Jiang W, Salamon J, Jücker M, Mautner VF
Int J Mol Sci 2020 Feb 24;21(4) doi: 10.3390/ijms21041548. PMID: 32102484Free PMC Article
Overcoming BET Inhibitor Resistance in Malignant Peripheral Nerve Sheath Tumors.
Cooper JM, Patel AJ, Chen Z, Liao CP, Chen K, Mo J, Wang Y, Le LQ
Clin Cancer Res 2019 Jun 1;25(11):3404-3416. Epub 2019 Feb 22 doi: 10.1158/1078-0432.CCR-18-2437. PMID: 30796033Free PMC Article
Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells.
Varin J, Poulain L, Hivelin M, Nusbaum P, Hubas A, Laurendeau I, Lantieri L, Wolkenstein P, Vidaud M, Pasmant E, Chapuis N, Parfait B
Oncotarget 2016 Jun 14;7(24):35753-35767. doi: 10.18632/oncotarget.7099. PMID: 26840085Free PMC Article

Prognosis

Ex vivo to in vivo model of malignant peripheral nerve sheath tumors for precision oncology.
Larsson AT, Bhatia H, Calizo A, Pollard K, Zhang X, Conniff E, Tibbitts JF, Rono E, Cummins K, Osum SH, Williams KB, Crampton AL, Jubenville T, Schefer D, Yang K, Lyu Y, Pino JC, Bade J, Gross JM, Lisok A, Dehner CA, Chrisinger JSA, He K, Gosline SJC, Pratilas CA, Largaespada DA, Wood DK, Hirbe AC
Neuro Oncol 2023 Nov 2;25(11):2044-2057. doi: 10.1093/neuonc/noad097. PMID: 37246765Free PMC Article
Systemic Options for Malignant Peripheral Nerve Sheath Tumors.
Hassan A, Pestana RC, Parkes A
Curr Treat Options Oncol 2021 Feb 27;22(4):33. doi: 10.1007/s11864-021-00830-7. PMID: 33641042
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406
Confirmation of mutation landscape of NF1-associated malignant peripheral nerve sheath tumors.
Sohier P, Luscan A, Lloyd A, Ashelford K, Laurendeau I, Briand-Suleau A, Vidaud D, Ortonne N, Pasmant E, Upadhyaya M
Genes Chromosomes Cancer 2017 May;56(5):421-426. Epub 2017 Mar 7 doi: 10.1002/gcc.22446. PMID: 28124441
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

Clinical prediction guides

Ex vivo to in vivo model of malignant peripheral nerve sheath tumors for precision oncology.
Larsson AT, Bhatia H, Calizo A, Pollard K, Zhang X, Conniff E, Tibbitts JF, Rono E, Cummins K, Osum SH, Williams KB, Crampton AL, Jubenville T, Schefer D, Yang K, Lyu Y, Pino JC, Bade J, Gross JM, Lisok A, Dehner CA, Chrisinger JSA, He K, Gosline SJC, Pratilas CA, Largaespada DA, Wood DK, Hirbe AC
Neuro Oncol 2023 Nov 2;25(11):2044-2057. doi: 10.1093/neuonc/noad097. PMID: 37246765Free PMC Article
Telomere alterations in neurofibromatosis type 1-associated solid tumors.
Rodriguez FJ, Graham MK, Brosnan-Cashman JA, Barber JR, Davis C, Vizcaino MA, Palsgrove DN, Giannini C, Pekmezci M, Dahiya S, Gokden M, Noë M, Wood LD, Pratilas CA, Morris CD, Belzberg A, Blakeley J, Heaphy CM
Acta Neuropathol Commun 2019 Aug 28;7(1):139. doi: 10.1186/s40478-019-0792-5. PMID: 31462295Free PMC Article
MEK inhibitors enhance therapeutic response towards ATRA in NF1 associated malignant peripheral nerve sheath tumors (MPNST) in-vitro.
Fischer-Huchzermeyer S, Dombrowski A, Wilke G, Stahn V, Streubel A, Mautner VF, Harder A
PLoS One 2017;12(11):e0187700. Epub 2017 Nov 13 doi: 10.1371/journal.pone.0187700. PMID: 29131833Free PMC Article
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

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